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BBOX1

Official Full Name
gamma-butyrobetaine hydroxylase 1
Organism
Homo sapiens
GeneID
8424
Background
This gene encodes gamma butyrobetaine hydroxylase which catalyzes the formation of L-carnitine from gamma-butyrobetaine, the last step in the L-carnitine biosynthetic pathway. Carnitine is essential for the transport of activated fatty acids across the mitochondrial membrane during mitochondrial beta-oxidation. [provided by RefSeq, Jul 2008]
Synonyms
BBH; BBOX; G-BBH; gamma-BBH;

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